[Prader-Willi syndrome and growth hormone treatment].
Prader Willi syndrome (PWS) is a hypothalamo-hypophyseal disorder associated with eating disorders, morbid obesity and behavioural troubles. A deletion of a segment of paternal chromosome 15 is the more frequent cause of PWS. The syndrome is associated with increased morbidity (sleep apnea, increased cardio-vascular risk) and mortality (mainly due to respiratory infectious diseases). GH secretion is usually decreased. GH treatment induces height gain, positive body composition changes and improves psychomotor development. Obstructive apnea was described in case of rapid increase in the dose of GH. Corticotroph deflciency, warranting treatment in stress situations could also take part in the high mortality rate of these patients.
Castinetti
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F
Service d'Endocrinologie, Diabète et Maladies Métaboliques, Hôpital de la Timone et Centre de Référence des Maladies Rares d'origine hypophysaire, Université de la Méditerranée, Marseille, France. CastiFred@free.fr
Reynaud
R
R
Brue
T
T
D001823:Body Composition; D002884:Chromosomes, Human, Pair 15; D005260:Female; D006130:Growth Disorders / Q000188:drug therapy / Q000209:etiology; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D011218:Prader-Willi Syndrome / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D011596:Psychomotor Disorders / Q000209:etiology; D012600:Scoliosis / Q000150:complications; D017384:Sequence Deletion; D012891:Sleep Apnea Syndromes / Q000453:epidemiology / Q000209:etiology
D019382:Human Growth Hormone